Respiratory manifestations, pulmonary function and high-resolution CT scan findings in common variable immune deficiency patients (CVID)

HAKL, Roman, Jiří VLČEK, Věra POSTRÁNECKÁ and Jiří LITZMAN. Respiratory manifestations, pulmonary function and high-resolution CT scan findings in common variable immune deficiency patients (CVID). In XXVI Congress of the European Academy of Allergology and Clinical Immunology. 2007. ISBN 0105-4538.

Basic information

• Original name: Respiratory manifestations, pulmonary function and high-resolution CT scan findings in common variable immune deficiency patients (CVID)
• Name in Czech: REspiorační manifestace, plicní funkce a nálezy HRCT u nemocných s běžným variabilním imunodeficitem (CVID)
• Authors: HAKL, Roman, Jiří VLČEK, Věra POSTRÁNECKÁ and Jiří LITZMAN.
• Edition: XXVI Congress of the European Academy of Allergology and Clinical Immunology, 2007.

Other information

• Original language: English
• Type of outcome: Presentations at conferences
• Field of Study: 30102 Immunology
• Country of publisher: Sweden
• Confidentiality degree: is not subject to a state or trade secret
• Organization unit: Faculty of Medicine
• ISBN: 0105-4538
• Keywords in English: Common variable immunodeficiency, lung fucntion, HRCT scan
• Tags: International impact

Abstract

Background: Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by low serum immunoglobulin levels and decreased specific antibody response after antigen challenge. Recurrent pyogenic infections caused by encapsulated bacteria of upper and lower respiratory tracts are the predominant clinical manifestation of CVID leading to various structural and functional abnormalities of the respiratory tract. The goal of our study was to assess pulmonary function and high-resolution computerized tomography scan in patients with CVID. Methods: Retrospective analysis of 48 patients with CVID followed-up since 1983 in our department was performed. The mean age of our group was 44,4 years, 6 of them died during the follow-up. The mean level of IgG in the time of diagnosis was 2,09 g/l. Pulmonary function were assessed by pulmonary function tests (PFTs) and high-resolution computerized tomography (HRCT). The airflow obstruction was graded by means of the Tiffeneau index. Metacholin provocation was used to detect bronchial hyperreactivity (BHRs) in 11 patients Results: The average delay of diagnosis was 6,8 years. The average number of pneumonias before the diagnosis was 2,3. Lung function test showed: obstructive changes in 26 patients (mild 17, moderate 5, severe 4); restrictive pattern in 7 patients (mild 5, moderate 2). Combined ventilation defect was observed in 6 patients. HRCT showed pulmonary abnormalities in 38 patients. Bronchiectasis were observed in 29 patients, granulomatosus lung disease in 3 patients and lung fibrosis in 19 patients. BHR was mild in 3 patients, moderate in 1 and severe in 1 patient. Surprisingly we did not find any correlations beween degree of lung obstruction and the length of diagnostic delay, referral IgG levels or number of pneumonias before the diagnosis. Conclusion: Pulmonary abnormalities develop in a proportion of patients with primary hypogammaglobulinemia. The development of airway lung abnormalities is individual. HRCT and PFTs are methods of choice in monitoring pulmonary abnormalities in hypogammaglobulinemic patients.

Abstract (in Czech)

Jsou popsány nálezy vyšetření plicních funkcí a HRCT plic u nemocných s běžným variabilním imunodeficitem.